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Adult Ependymal Tumor

MedGen UID:
323813
•Concept ID:
C1541566
•
Neoplastic Process

Definition

An ependymal tumor of the central nervous system occurring in adults. [from NCI]

Professional guidelines

PubMed

Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free PMC Article
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article
Recent Advances in the Classification and Treatment of Ependymomas.
Leeper H, Felicella MM, Walbert T
Curr Treat Options Oncol 2017 Aug 10;18(9):55. doi: 10.1007/s11864-017-0496-7. PMID: 28795287

Recent clinical studies

Etiology

Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free PMC Article
Central nervous system gliomas.
Reni M, Mazza E, Zanon S, Gatta G, Vecht CJ
Crit Rev Oncol Hematol 2017 May;113:213-234. Epub 2017 Mar 22 doi: 10.1016/j.critrevonc.2017.03.021. PMID: 28427510
Glioma.
Weller M, Wick W, Aldape K, Brada M, Berger M, Pfister SM, Nishikawa R, Rosenthal M, Wen PY, Stupp R, Reifenberger G
Nat Rev Dis Primers 2015 Jul 16;1:15017. doi: 10.1038/nrdp.2015.17. PMID: 27188790
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free PMC Article
Ependyma-lined cysts.
Williams Z, Herrick MK, Tse V
Clin Neuropathol 2000 May-Jun;19(3):138-41. PMID: 14606587

Diagnosis

Glioma.
Weller M, Wen PY, Chang SM, Dirven L, Lim M, Monje M, Reifenberger G
Nat Rev Dis Primers 2024 May 9;10(1):33. doi: 10.1038/s41572-024-00516-y. PMID: 38724526
Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free PMC Article
Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.
Horbinski C, Berger T, Packer RJ, Wen PY
Nat Rev Neurol 2022 Sep;18(9):515-529. Epub 2022 Jun 21 doi: 10.1038/s41582-022-00679-w. PMID: 35729337
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article
Central nervous system gliomas.
Reni M, Mazza E, Zanon S, Gatta G, Vecht CJ
Crit Rev Oncol Hematol 2017 May;113:213-234. Epub 2017 Mar 22 doi: 10.1016/j.critrevonc.2017.03.021. PMID: 28427510

Therapy

Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.
Horbinski C, Berger T, Packer RJ, Wen PY
Nat Rev Neurol 2022 Sep;18(9):515-529. Epub 2022 Jun 21 doi: 10.1038/s41582-022-00679-w. PMID: 35729337
Recent Advances in the Classification and Treatment of Ependymomas.
Leeper H, Felicella MM, Walbert T
Curr Treat Options Oncol 2017 Aug 10;18(9):55. doi: 10.1007/s11864-017-0496-7. PMID: 28795287
Chemotherapy for intracranial ependymoma in adults.
Gramatzki D, Roth P, Felsberg J, Hofer S, Rushing EJ, Hentschel B, Westphal M, Krex D, Simon M, Schnell O, Wick W, Reifenberger G, Weller M
BMC Cancer 2016 Apr 23;16:287. doi: 10.1186/s12885-016-2323-0. PMID: 27108407Free PMC Article
Adult ependymal tumors: prognosis and the M. D. Anderson Cancer Center experience.
Armstrong TS, Vera-Bolanos E, Bekele BN, Aldape K, Gilbert MR
Neuro Oncol 2010 Aug;12(8):862-70. Epub 2010 Feb 5 doi: 10.1093/neuonc/noq009. PMID: 20511182Free PMC Article
Ependymal tumors.
Moynihan TJ
Curr Treat Options Oncol 2003 Dec;4(6):517-23. doi: 10.1007/s11864-003-0052-5. PMID: 14585232

Prognosis

Glioma.
Weller M, Wen PY, Chang SM, Dirven L, Lim M, Monje M, Reifenberger G
Nat Rev Dis Primers 2024 May 9;10(1):33. doi: 10.1038/s41572-024-00516-y. PMID: 38724526
EANO guidelines for the diagnosis and treatment of ependymal tumors.
Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC
Neuro Oncol 2018 Mar 27;20(4):445-456. doi: 10.1093/neuonc/nox166. PMID: 29194500Free PMC Article
Central nervous system gliomas.
Reni M, Mazza E, Zanon S, Gatta G, Vecht CJ
Crit Rev Oncol Hematol 2017 May;113:213-234. Epub 2017 Mar 22 doi: 10.1016/j.critrevonc.2017.03.021. PMID: 28427510
Glioma.
Weller M, Wick W, Aldape K, Brada M, Berger M, Pfister SM, Nishikawa R, Rosenthal M, Wen PY, Stupp R, Reifenberger G
Nat Rev Dis Primers 2015 Jul 16;1:15017. doi: 10.1038/nrdp.2015.17. PMID: 27188790
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free PMC Article

Clinical prediction guides

Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment.
Träger M, Schweizer L, Pérez E, Schmid S, Hain EG, Dittmayer C, Onken J, Fukuoka K, Ichimura K, Schüller U, Dührsen L, Müther M, Paulus W, Thomas C, Gutt-Will M, Schucht P, Maragkou T, Schittenhelm J, Eckert F, Niyazi M, Fleischmann DF, Dorostkar MM, Feyer P, May SA, Moskopp D, Badakhshi H, Radke C, Walter J, Ehret F, Capper D, Kaul D
Neuro Oncol 2023 Jul 6;25(7):1286-1298. doi: 10.1093/neuonc/noad030. PMID: 36734226Free PMC Article
Adult Primary Brain Neoplasm, Including 2016 World Health Organization Classification.
Wang KY, Chen MM, Malayil Lincoln CM
Neuroimaging Clin N Am 2021 Feb;31(1):121-138. doi: 10.1016/j.nic.2020.09.011. PMID: 33220825
Genomic Molecular Classification of CNS Malignancies.
Spino M, Snuderl M
Adv Anat Pathol 2020 Jan;27(1):44-50. doi: 10.1097/PAP.0000000000000254. PMID: 31714292
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups.
Pajtler KW, Witt H, Sill M, Jones DT, Hovestadt V, Kratochwil F, Wani K, Tatevossian R, Punchihewa C, Johann P, Reimand J, Warnatz HJ, Ryzhova M, Mack S, Ramaswamy V, Capper D, Schweizer L, Sieber L, Wittmann A, Huang Z, van Sluis P, Volckmann R, Koster J, Versteeg R, Fults D, Toledano H, Avigad S, Hoffman LM, Donson AM, Foreman N, Hewer E, Zitterbart K, Gilbert M, Armstrong TS, Gupta N, Allen JC, Karajannis MA, Zagzag D, Hasselblatt M, Kulozik AE, Witt O, Collins VP, von Hoff K, Rutkowski S, Pietsch T, Bader G, Yaspo ML, von Deimling A, Lichter P, Taylor MD, Gilbertson R, Ellison DW, Aldape K, Korshunov A, Kool M, Pfister SM
Cancer Cell 2015 May 11;27(5):728-43. doi: 10.1016/j.ccell.2015.04.002. PMID: 25965575Free PMC Article
Ovarian ependymoma.
Erdoğan G, Ozel E, Peştereli HE, Salar Z, Tirak B, Karaveli S
APMIS 2005 Apr;113(4):301-3. doi: 10.1111/j.1600-0463.2005.apm_10.x. PMID: 15865613

Recent systematic reviews

Intracranial glioependymal (neuroglial) cysts: a systematic review.
Robles LA, Paez JM, Ayala D, Boleaga-Duran B
Acta Neurochir (Wien) 2018 Jul;160(7):1439-1449. Epub 2018 May 25 doi: 10.1007/s00701-018-3566-0. PMID: 29802560

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